title: Rhinoscleroma: a French national retrospective study of epidemiological and clinical features.
abstract: BACKGROUND
  Rhinoscleroma (RS) is a rare, chronic, granulomatous disease of the upper respiratory tract that is associated with infection with Klebsiella rhinoscleromatis. RS is more common in certain geographic regions than in others, but other risk factors and the pathogenesis of RS remain unclear.
  
  
  METHODS
  We sent a standardized questionnaire to all pathologists and otolaryngology specialists in French University Hospitals and asked whether they had seen patients with RS in the previous 16 years (1990-2005). We then retrospectively reviewed the files of all patients identified.
  
  
  RESULTS
  We collected 11 cases of RS, with a median patient age at diagnosis of 35.7 years (range, 5-72 years). The 3 patients with a familial history of RS presented with early-onset forms of RS; 1 had an uncommon aggressive presentation of the disease with ischemic stroke. Two unrelated consanguineous families were identified, 1 of which included 2 affected siblings. Two patients with sporadic disease were positive for HIV infection. All patients were living in France, but most were immigrants from areas where RS is endemic (North Africa, 3 of the 11 patients; West Africa, 4 patients; and Turkey, 1 patient). The probable duration of exposure to K. rhinoscleromatis in endemic areas varied widely: 0-28 years. Clinical features and outcome also varied considerably among cases. Biopsies had been performed for all patients and revealed granulomas containing Mikulicz cells. Cultures of biopsy tissue were positive for K. rhinoscleromatis in 5 of the 11 cases. Prolonged antibiotic treatment was administered to all patients, as follows: ciprofloxacin (7 patients), third-generation cephalosporins (2), tetracycline (2), and clofazimine (2). Eight of the 11 patients did not experience relapse during extended periods of follow-up (1.3-12 years). Relapses in 3 patients were confirmed by a second biopsy.
  
  
  CONCLUSIONS
  The occurrence of early-onset RS in multiplex and/or consanguineous families suggests that genetic control of the host response to K. rhinoscleromatis may be involved in the pathogenesis of RS in endemic areas.
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Service de Pédiatrie, Université Paris 13, Hôpital Jean Verdier, Bondy, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: de Pontual
authors.forename: Philippe
authors.initials: P
authors.lastname: Ovetchkine
authors.forename: Diana
authors.initials: D
authors.lastname: Rodriguez
authors.forename: Audrey
authors.initials: A
authors.lastname: Grant
authors.forename: Anne
authors.initials: A
authors.lastname: Puel
authors.forename: Jacinta
authors.initials: J
authors.lastname: Bustamante
authors.forename: Sabine
authors.initials: S
authors.lastname: Plancoulaine
authors.forename: Laurent
authors.initials: L
authors.lastname: Yona
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Lienhart
authors.forename: Danièle
authors.initials: D
authors.lastname: Dehesdin
authors.forename: Michel
authors.initials: M
authors.lastname: Huerre
authors.forename: Régis
authors.initials: R
authors.lastname: Tournebize
authors.forename: Philippe
authors.initials: P
authors.lastname: Sansonetti
authors.forename: Laurent
authors.initials: L
authors.lastname: Abel
authors.forename: Jean Laurent
authors.initials: JL
authors.lastname: Casanova
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002675:Child, Preschool; D054242:Emigrants and Immigrants; D005260:Female; D005602:France / Q000453:epidemiology; D006099:Granuloma / Q000473:pathology; D006785:Hospitals, University; D006801:Humans; D007711:Klebsiella pneumoniae / Q000302:isolation & purification; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012226:Rhinoscleroma / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology*; D012307:Risk Factors; D011795:Surveys and Questionnaires
chemical_list: D000900:Anti-Bacterial Agents